Cerebellar presence of immune cells in patients with neuro-coeliac disease.

Although various neurodegenerative disorders have been associated with coeliac disease (CD), the underlying neuropathological link between these brain and gut diseases remains unclear. We postulated that the neuronal damage sporadically observed in CD patients is immune-mediated. Our aim was to determine if the loss of neurons, especially Purkinje cells, coincides with microglia activation and T- and B-cell infiltration in the cerebellum of patients with CD and a concomitant idiopathic neurological disease affecting the cerebellum (NeuroCD). Post-mortem cerebellar tissue was collected of validated NeuroCD cases. Gender- and age-matched genetic spinocerebellar ataxia (SCA) controls and non-neurological controls (NNC) were selected based on clinical reports and pathological findings. Cerebellar tissue of seventeen patients was included (6 NeuroCD, 5 SCA, 6 NNC). In SCA cases we found that the Purkinje cell layer was 58.6% reduced in comparison with NNC. In NeuroCD cases this reduction was even more prominent with a median reduction of 81.3% compared to NNC. Marked increased numbers of both CD3+ and CD8+ cells were observed in the NeuroCD but not in SCA patients. This coincided with significantly more microglial reactivity in NeuroCD patients. These findings demonstrate that the massive loss of Purkinje cells in the cerebellum of neuro CD patients is accompanied by local innate and T-cell mediated immune responses.

Subacromial impingement by a lipoma arborescens.

Subacromial impingement syndrome (SIS) is the leading cause of shoulder pain. A systemic approach for abnormal causes of SIS is recommended to avoid misdiagnosing rare or sinister pathologies. To our knowledge, only nine cases of subacromial lipoma arborescens associated with impingement syndrome have been reported in the literature. In this report, we briefly discuss histopathologic and radiological signs of an unusual case of impingement syndrome caused by subacromial "lipoma arborescens" and describe arthroscopic synovectomy after the failure of conservative management. The patient remains symptom-free five years after surgery.

Challenges in diagnosing aortic leiomyosarcoma post endovascular repair of abdominal aortic aneurysm.

Primary aortic tumors after endovascular aortic repair are rarely reported in the literature. Here, we report an elderly male with abdominal aortic leiomyosarcomas (LMS) after an endovascular aneurysm repair in 2012 for a 5-cm symptomatic abdominal aortic aneurysm using an Endurant II aortic stent graft (Medtronic, Minneapolis, Minn). The autopsy confirmed the aortic LMS after the patient rapidly deteriorated and succumbed to death. The vascular LMS are rapidly progressive and diagnostically challenging malignant soft tissue tumors with poor prognosis, which necessitates a strong clinical suspicion and attentiveness to radiologic signs for prompt diagnosis.

The adequacy of lymph node clearance in colon cancer surgery performed in a non-specialist centre; implications for practice.

BACKGROUND: Despite recent medical advances, surgery remains the mainstay treatment in colon cancer. It is well established that better patient outcomes are achieved when complex surgery including pancreatic, oesophageal and rectal surgeries are carried out in high-volume centres. However, it is unclear as to whether or not colon cancer patients receive the same benefit. Lymph node adequacy is a key performance indicator of successful oncological colonic resection which impacts on patient outcome. AIM: To assess the adequacy of lymph node clearance during colonic resection performed with curative intent in a non-specialist centre post introduction of the National Cancer Strategy. METHODS: Retrospective analysis was performed of a prospectively maintained database examining the lymph node clearance of all oncological resections for colon cancer over a 7-year period (Nov 2010-Dec 2017) at a satellite unit with links to a regional specialist centre. Primary outcome measured was the number of lymph nodes retrieved. Secondary outcomes included resection margins, 30-day complication rate and survival at 1 year. Statistical analysis was performed using SPSS Statistics for Windows, version 24.0 (IBM Corp, Armonk, N.Y., USA). RESULTS: One hundred sixty-seven patients were included. Mean age was 71.0 ± 11.6 years. Majority were male (n = 90, 53.6%). The majority of resections was right sided (n = 112.66.7%) with 78.6% of all resections being undertaken electively. All margins were free of tumour. The average lymph node count was 19.93 ± 8.63 (4.62) with only 17 (10.2%) of specimens containing < 12 nodes. The anastomotic leak rate was 3.3%. There was no association between surgeon or pathologist volume, nor emergent status and achieving oncological lymph node count (p = 0.14, 0.29, 0.97). 90.5% of patients were alive at 1 year. CONCLUSIONS: This study demonstrates that colonic cancer surgery can be safely performed in a non- specialist centre with technical outcomes comparable to nationally reported figures.

What is the cause of this synchronous palpable abdominal mass in a woman recently diagnosed with lung cancer as demonstrated in Figure 1?

Colonic metastases from lung cancer are rare [1, 2]. Presentation of an abdominal mass in the setting of a new lung cancer diagnosis should prompt complete evaluation including endoscopic and CT imaging. This case also highlights the need for immunohistochemical analysis of unusual tumor deposits facilitating appropriate treatment.

Paediatric Ewing-like sarcoma arising from the cranium - a unique diagnostic challenge.

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Predictive factors for colonic resection in patients less than 49 years with symptomatic diverticular disease.

BACKGROUND: Diverticular disease is a condition strongly associated with low-fiber intake and obesity. There have been reports of an increasing incidence in younger individuals ranging from 12% to 21% of all cases. The aim of this study was to evaluate the management of complicated diverticular disease in patients less than 49 years and attempt to identify factors predictive of a more virulent course. METHODS: An analysis of a prospectively updated database of all patients admitted with a primary diagnosis of acute diverticulitis from 2005 to 2013 was performed. Data collected included age, length of stay, inflammatory markers on admission, use of computed tomography (CT), and Hinchey Classification. SPSS version 22 was used for statistical analysis, and a P value of .05 or less was considered significant. RESULTS: A total of 120 (54 female and 66 male) patients less than 49 (28 to 49, 42.1) years were noted to have a diagnosis of acute diverticulitis. Twelve patients (10%) required colonic resection for complicated diverticulitis. Histological evaluation revealed 5 cases of stricture, 2 obstruction, and 5 perforations. On multivariate analysis, predictors of operative intervention and/or colonic resection included, (hazard ratio [95% confidence interval]) patients aged 40 to 49 years (.92 [.9 to .95]) and elevated C-reactive protein on index admission (1.4 [1.32 to 1.54]). Females were less likely to undergo colonic resection compared with males (1.18 [1.15 to 1.2]). Median length of stay was 4 days (1 to 48) for patients managed nonoperatively and 13 days (5 to 27) for those who underwent surgery. CONCLUSIONS: Most younger patients with acute diverticulitis can be treated successfully by conservative means. However, a proportion of patients require aggressive surgical management.

Malignant neuroendocrine tumour of the appendix in childhood with loco-regional lymph node invasion.

VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1006600359152743.

Isolated cutaneous metastasis of uterine leiomyosarcoma: case report and review of literature.

A 54 year old lady presented for routine excision of a scalp lesion thought clinically to represent a sebaceous cyst of the right occiput. 4 years earlier she underwent total abdominal hysterectomy and right salpingo-oophorectomy for 3 large uterine fibroids. Histo-pathological examination of the hysterectomy specimen revealed an incidental low-grade leiomyosarcoma. Staging imaging was negative for metastatic disease. She made an uneventful recovery and was treated further by adjuvant pelvic radiotherapy.She noticed an uncomfortable and unsightly cystic swelling on her occiput four years after hysterectomy and was referred for routine excision of what was believed to be a benign lesion. The lesion was excised and sent for histopathological examination. Microscopic analysis including immuno-histochemistry demonstrated an ER and PR positive metastatic deposit of leiomyosarcoma. The margins of excision were histologically clear of disease.At Multi-Disciplinary Team (MDT) discussion a diagnosis of metastatic scalp deposit from previous uterine leiomyosarcoma was made. Re-staging CT brain, thorax, abdomen and pelvis and MRI brain were negative for local recurrence or distant metastases. She is currently undergoing radiotherapy to the scalp and surrounding tissues and will be followed up closely by the involved teams.To the best of our knowledge, this is the first case described in the worldwide literature of isolated cutaneous metastasis to the scalp of uterine leiomyosarcoma without evidence of disseminated disease at other sites. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1311834987345566.

Is overexpression of HER-2 a predictor of prognosis in colorectal cancer?

BACKGROUND: The development of novel chemotherapeutic agents in colorectal cancer has improved survival. Following initial response to chemotherapeutic strategies many patients develop refractory disease. This poses a significant challenge common to many cancer subtypes. Newer agents such as Bevacizumab have successfully targeted the tyrosine kinase receptor epidermal growth factor receptor in metastatic colorectal cancer. Human epidermal growth factor receptor-2 is another member of the tyrosine kinase receptor family which has been successfully targeted in breast cancer. This may play a role in colorectal cancer. We conducted a clinicopathological study to determine if overexpression of human epidermal growth factor receptor-2 is a predictor of outcome in a cohort of patients with colorectal cancer. METHODS: Clinicopathological data and paraffin-embedded specimens were collected on 132 consecutive patients who underwent colorectal resections over a 24-month period at Mayo General Hospital. Twenty-six contained non-malignant disease. Her-2/neu protein overexpression was detected using immunohistochemistry (IHC). The HER-2 4B5 Ventana monoclonal antibody was used. Fluorescent insitu hybridisation (FISH) was performed using INFORM HER-2/Neu Plus. Results were correlated with established clinical and pathological predictors of outcome including TNM stage. Statistical analysis was performed using SPSS version 11.5. RESULTS: 114 were HER-2/Neu negative using IHC, 7 showed barely perceptible positivity (1+), 9 showed moderate staining (2+) and 2 were strongly positive (3+). There was no correlation with gender, age, grade, Dukes' stage, TNM stage, time to recurrence and 5-year survival (p > 0.05). FISH was applied to all 2+ and 3+ cases as well as some negative cases selected at random. Three were amplified (2 were 3+ and 1 was 2+). Similarly, HER-2 gene overexpression did not correlate with established prognostic indicators. CONCLUSION: HER-2 protein is over expressed in 11% of colorectal cancer patients. The gene encoding HER-2 is amplified in 3% of cases. Overexpression of HER-2 is not a predictor of outcome. However, patients who over express HER-2 may respond to Herceptin therapy.

Enteropathy associated T cell lymphoma: common in coeliac disease.

A 56-year-old male admitted with haematemesis and epigastric pain and severe weight loss on a background of coeliac disease. Computed tomography (CT) abdomen revealed a thickening of the mucosal folds of a short segment of jejunum. He deteriorated and had an exploratory laparotomy and bowel resection with side-side jejojejunal stapled anastomosis and extended right hemicolectomy and ileocolic anastomosis. Histology demonstrated multifocal high-grade malignant T cell lymphoma. Coeliac disease is a very common lifelong disorder. It is associated with osteoporosis, infertility, autoimmune disorders and increased risk of malignancy including an increased risk of non-Hodgkin's lymphoma (NHL) especially of the T cell type. Enteropathy-type T cell lymphoma is associated with a very poor prognosis. There is significant evidence that adherence to a gluten-free diet decreases the risk of developing enteropathy-type T cell lymphoma and helps to prevent development of autoimmune diseases, diabetes mellitus and osteoporosis in patients with coeliac disease.